[Isolated isoleucine deficiency in diet therapy of a case of maple syrup urine disease].

A case of maple syrup urine disease is described presenting during diet therapy a clinical picture with predominant cutaneous symptoms attributed to a dietary deficiency. It is believed that probable cause was the coexistence of low serum levels of isoleucine with still elevated levels of leucine. Given the metabolic interrelationship existing between different branch-chained amino acids, an independent dietary correction of each amino acid is recommended.